Richter's syndrome: a report on 39 patients.

Robertson, L. E.; Pugh, William; O’Brien, Susan; Kantarjian, Hagop M.; Hirsch-Ginsberg, Cheryl F; Cork, Ann; McLaughlin, Peter; Cabanillas, Fernando; Keating, Michael J.

doi:10.1200/jco.1993.11.10.1985

Cited by 236 publications

(226 citation statements)

References 20 publications

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“…Histological progression to large-cell lymphoma also corresponds to a general clinical alteration, as observed in our patients (1,2,12). Transformation occurs in lymph nodes, bone marrow, spleen, or other tissues (1, 2, 12).…”

Section: Discussionsupporting

confidence: 75%

“…This process is commonly referred to as Richter's syndrome (RS; 9, 10). RS is characterized by rapid lymph node enlargement, splenomegaly, hepatomegaly, fever, and weight loss and is associated with a rapid fatal outcome (1,2,9,11,12). Because patients with CLL have a greater tendency to develop secondary tumors, there has been a debate about the origin of the DLCL.…”

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confidence: 99%

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Primary Digestive Richter's Syndrome

et al. 2001

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The clinical and morphologic transformation of 3 to 5% of chronic lymphocytic leukemia (CLL) to diffuse large-cell lymphoma (DLCL) is commonly referred to as Richter's syndrome. Richter's syndrome occurs mostly in lymph nodes and may represent a second neoplasm or a transformation from the same clonal population. Clinical features in six patients with digestive Richter's syndrome were recorded. Paired samples of CLL and DLCL were investigated by immunohistological analysis (n ‫؍‬ 6) and by polymerase chain reaction (PCR) for immunoglobulin heavy-chain gene rearrangement (n ‫؍‬ 4). Histological examination revealed the involvement of the gastrointestinal tract by DLCL of B-cell phenotype (n ‫؍‬ 6). The same monoclonal rearrangement between CLL and DLCL was demonstrated by PCR and sequencing analyses in two patients. The monoclonal rearrangement was different between CLL and DLCL in only one case. Median survival was 22 months for five patients receiving chemotherapy, suggesting that digestive Richter's syndrome has a better prognosis than nodal Richter's syndrome. Indeed, appropriate surgical resection combined with chemotherapy led to partial or complete remission in four patients.

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Section: Discussionsupporting

confidence: 75%

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confidence: 99%

Primary Digestive Richter's Syndrome

et al. 2001

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“…It is usually characterized by sudden clinical deterioration as manifest by systemic symptoms such as fever, night sweats, weight loss, increase in lymphadenopathy and hepatosplenomegaly, or extranodal involvement [3,4]. Recent studies show that the incidence of RS in CLL/SLL is approximately 3% [5,6]; however, development of high-grade lymphoma following MCL has only rarely been reported [1,7].…”

Section: Introductionmentioning

confidence: 99%

Case of Epstein‐Barr virus‐associated transformation of mantle cell lymphoma

Terasawa¹,

Ohashi²,

Utsumi³

et al. 2003

American J Hematol

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“…1 Moreover, those patients who respond well to treatment may enjoy prolonged survival. 3 Recently, allogeneic stem cell transplantation (SCT) has become a new strategy for the treatment of patients with RS, and preliminary results suggest that this may improve the prognosis when compared with conventional chemotherapy. 4 We report a case of high-grade NHL that appeared 4 months after allogeneic SCT for chronic lymphatic leukaemia (CLL), jointly with the reappearance of the original low-grade malignancy.…”

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confidence: 99%

“…Clinical deterioration is accompanied by a shortened survival. 3 Treatment modalities for RS include systemic chemotherapy with fludarabine-containing regimens, CHOPbased regimens, or other chemotherapy schedules used for high-grade lymphomas, such as MACOP-B and ASHAP, with or without adjuvant radiotherapy. Overall response rate in the patient group described by Robertson et al 3 was 42%, and the median survival was 5 months.…”

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confidence: 99%

Richter's syndrome after allogeneic stem cell transplantation for chronic lymphocytic leukaemia successfully treated by withdrawal of immunosuppression, and donor lymphocyte infusion

Español

Büchler

Ferrà

et al. 2003

Bone Marrow Transplant

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Summary:Development of high-grade non-Hodgkin's lymphoma is a possible complication of chronic lymphocytic leukaemia/ small lymphocytic lymphoma, known as Richter's syndrome (RS). Treatment for RS includes systemic chemotherapy and, recently, allogeneic stem cell transplantation (SCT). We describe a patient with B-chronic lymphocytic leukaemia who developed RS 4 months after allogeneic SCT from an HLA-identical sibling. The RS presented with systemic symptoms, lymphadenopathy, pancytopenia and serum lactate dehydrogenase elevation. The patient was treated with immunosuppressive drug withdrawal and a donor lymphocyte infusion (DLI) of 1 Â 10 7 CD3/kg, leading to the disappearance of all symptoms and the attainment of complete donor chimerism. After 18 months of the therapeutic DLI, the patient continues in complete remission.

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Richter's syndrome: a report on 39 patients.

Cited by 236 publications

References 20 publications

Primary Digestive Richter's Syndrome

Primary Digestive Richter's Syndrome

Case of Epstein‐Barr virus‐associated transformation of mantle cell lymphoma

Richter's syndrome after allogeneic stem cell transplantation for chronic lymphocytic leukaemia successfully treated by withdrawal of immunosuppression, and donor lymphocyte infusion

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